0000000000121597

AUTHOR

Martin P Hansen

Das polyglanduläre Autoimmunsyndrom – Lebensqualität und familiäre Beteiligung

Hintergrund und Fragestellung: Fur Patienten mit einem Polyglandularem Autoimmunsyndrom (PGA) und ihre Angehorigen liegen keine Daten zur familiaren Beteiligung und zur Lebensqualitat vor. Daher erfolgte eine Erhebung in einer reprasentativen Gruppe. Patienten und Methoden: Im Rahmen einer prospektiv angelegten und kontrollierten Studie wurden klinische und serologische Untersuchungen an 75 konsekutiv aufgenommenen Patienten mit PGA (mittleres Alter 47,5  ±  15,3 Jahre; 65,3 % Frauen) mit 108 Angehorigen (mittleres Alter 33,13 Jahre  ± 20,08 Jahre; 65,7% Frauen) durchgefuhrt. Drei validierte Messinstrumente (Short Form 36 [SF-36], Hospital Anxiety and Depression Scale [HADS] und Giesener Be…

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Type 1 diabetes associated autoimmunity.

Diabetes mellitus is increasing in prevalence worldwide. The economic costs are considerable given the cardiovascular complications and co-morbidities that it may entail. Type 1 diabetes (T1D) is a chronic autoimmune disease characterized by the loss of insulin-producing pancreatic β-cells. The pathogenesis of T1D is complex and multifactorial and involves a genetic susceptibility that predisposes to abnormal immune responses in the presence of ill-defined environmental insults to the pancreatic islets. Genetic background may affect the risk for autoimmune disease and patients with T1D exhibit an increased risk of other autoimmune disorders such as autoimmune thyroid disease, Addison's dise…

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HLA Class II Differentiates Between Thyroid and Polyglandular Autoimmunity.

The HLA class II genes are susceptibility genes for autoimmune endocrine diseases; however, scarce data are available pertaining to the determinants of genetic susceptibility to polyglandular autoimmunity (PGA). A total of 300 consecutive and unselected patients with either PGA or monoglandular autoimmune thyroid disease (AITD) and 100 healthy control subjects were genotyped for the HLA class II DRB1, -DQA1, and -DQB1 alleles. Compared to patients with AITD and controls, the HLA-DRB1*03 (pc =0.001), *04 (pc<0.001), -DQA1*03 (pc<0.001), and -DQB1*02 (pc =0.001) alleles were increased in patients with PGA. When dividing patients with Hashimoto's thyroiditis (HT) into those with PGA (PGA-HT) v…

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Type 1 diabetes and polyglandular autoimmune syndrome: A review

Type 1 diabetes (T1D) is an autoimmune disorder caused by inflammatory destruction of the pancreatic tissue. The etiopathogenesis and characteristics of the pathologic process of pancreatic destruction are well described. In addition, the putative susceptibility genes for T1D as a monoglandular disease and the relation to polyglandular autoimmune syndrome (PAS) have also been well explored. The incidence of T1D has steadily increased in most parts of the world, especially in industrialized nations. T1D is frequently associated with autoimmune endocrine and non-endocrine diseases and patients with T1D are at a higher risk for developing several glandular autoimmune diseases. Familial cluster…

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Osteosynthetische versorgung proximaler extraartikul�rer tibiafrakturen Indikationen, komplikationen, ergebnisse

In den letzten funf Jahren wurden 16 extraartikulare Frakturen der proximalen Tibia in der Unfallchirurgischen Universitatsklinik Mainz operativ versorgt. Es handelte sich dabei um proximale metaphysare und meta-/diaphysare Verletzungen ohne Beteiligung des Kniegelenkes. Eine begleitende Fraktur der Fibula lag bei 13 Patienten vor. Die Frakturen wurden nach dem AO-System klassifiziert. Es handelte sich um vier offene und zwolf geschlossene Frakturen. Abhangig von der Weichteilsituation wurde der Zeitpunkt der operativen Versorgung (im Mittel drei Tage posttraumatisch) gewahlt. Sechs Frakturen wurden mit einer intramedullaren Osteosynthese und zehn Frakturen mittels Plattenosteosynthese nach…

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Sex Alters the MHC Class I HLA-A Association With Polyglandular Autoimmunity.

Abstract Context The major histocompatibility complex (MHC) strongly contributes to the development of polyglandular autoimmunity (PGA). Objective To evaluate the impact of sex on human leukocyte antigen (HLA) association with PGA for the first time. Design Cross-sectional immunogenetic study. Setting Academic tertiary referral Orphan Disease Center for PGA (ORPHA 282196) and immunogenetics laboratory. Subjects Patients (158) with coexistent type 1 diabetes and autoimmune thyroid disease (adult type 3 PGA, ORPHA 227982) and 479 unrelated healthy controls. Interventions All 637 white subjects were typed for HLA-A, -B, -DRB1, -DQA1, and -DQB1 alleles at a two-field level. Main Outcome Measure…

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